title: Precocious puberty in children with myelomeningocele: treatment with gonadotropin-releasing hormone analogues.
abstract: In patients with myelomeningocele (MMC), growth is influenced by a large number of growth-retarding factors due to the neurological defect. Moreover, endocrine disorders have been found to contribute to short stature in MMC patients. Central precocious puberty (CPP) is a common problem. Due to growth disturbances and difficulties in obtaining standardized measurements, MMC patients have been excluded from gonadotropin-releasing hormone (GnRH) analogue studies in the past. We report on eight patients (six female, two male) with MMC, hydrocephalus, and CPP who were treated with GnRH analogues: triptorelin intramuscularly (N=5) or leuprorelin subcutaneously (N=3). Auxological data and hormone levels were assessed before treatment and every 6 months during treatment. The median chronological ages (CA) at the start of treatment were 8.6 years (females) and 8.4 years (males). Bone age (BA) was accelerated in all cases prior to treatment and two girls were already menstruating. Elevated gonadotropin serum levels and sex steroid levels decreased during treatment, although no complete suppression to prepubertal levels was reached. Progression of pubertal development and menses stopped in all patients. The tempo of BA acceleration (deltaBA:deltaCA) decreased, but no significant improvement in height standard deviation score BA and predicted adult height resulted. No side effects during treatment were observed. CPP in MMC patients has to be considered as early as possible to enable an early diagnosis and corresponding treatment. Further prospective studies on the effects of GnRH analogues in MMC patients are necessary.
authors.affiliation: University Hospital for Children and Adolescents, University of Erlangen-Nürnberg, Erlangen, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Trollmann
authors.forename: E
authors.initials: E
authors.lastname: Strehl
authors.forename: H G
authors.initials: HG
authors.lastname: Dörr
mesh_terms: D000365:Age Determination by Skeleton; D002648:Child; D005260:Female; D006062:Gonadotropins / Q000032:analysis; D006801:Humans; D006849:Hydrocephalus / Q000150:complications*; D016729:Leuprolide / Q000008:administration & dosage / Q000627:therapeutic use*; D008186:Luteolytic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008591:Meningomyelocele / Q000150:complications*; D008598:Menstruation / Q000187:drug effects; D011629:Puberty, Precocious / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D017329:Triptorelin Pamoate / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D006062:Gonadotropins; D008186:Luteolytic Agents; D017329:Triptorelin Pamoate; D016729:Leuprolide